We met with Dr. Roy yesterday, Hannah’s ENT. He is one of those doctors that you just feel a trust with, someone that is going to tell it to you straight, but also willing to actively “ride the ride” with us. Dr. B., our pediatrician, searched him out for us after our first ENT wanted us to proactively trach Hannah back when she was 11 months old because of her laryngomalacia and her prognosis.

We starting seeing Dr. Roy back in September of 2009, and yesterday was the third visit with him.   He noticed pretty significant laryngomalacia and some vocal cord weakness when he did his first laryngoscope (camera down Hannah’s nose while she was awake) as well as moderate reflux.   Back then, he heard the stridor sounds Hannah made when she was upset and the breathing issues when she was upset.  The trach was always in the picture, but we were able to walk away from the two previous visits with “We will review where we are in two months.”

Then we had Hannah’s multiple gagging/breathing episodes, two of which landed us with 911 at the ER.  I thought for sure we were close to having to make the trach decision.

We stopped solids and put Hannah on a liquid diet.  Not a single gagging episode in the 6 weeks that we started that.  Not even a minor one!

So when Dr. Roy came in yesterday, Hannah yelled at him.  He actually loved hearing that because he had never heard a strong vocal from her before.  We explained that she does now yell and has a stronger cry these days.  He also thought she looked really good too, which she does.

He did another laryngoscope today, and Hannah was a trooper.  This was her 5th time having that dang camera put down her nose into her throat.  But the results were GREAT!   He was so pleasantly shocked and surprised that Hannah’s laryngomalacia has significantly IMPROVED since our last visit plus her vocal cords were much more active — the vocal cord weakness has IMPROVED as well!  Her reflux is still there but definitely looks better.

We never expected an improvement in Hannah’s symptoms, especially the ones that are so closely tied to her Gaucher’s disease.  But it happened.  Her laryngomalacia and her vocal cord weakness have definitely IMPROVED.  Say it with me, IMPROVED!!  I love saying that!!!

We all agreed that we would rather have to deal with a gastric tube in feeding Hannah if it comes to that than having to deal with a tracheotomy.  For whatever reason, a G-tube just does not seem anywhere near as drastic as a trach.  Dr. Roy also agreed that a G-tube is much easier and safer to deal with than a trach.  But we are not at a G-tube yet, as long as she keeps her weight and keeps growing, we are going to stick to bottle feeding.

So all of the breathing/gagging/feeding issues we have been dealing with for the past many months have been all related to her hypersensitive defensive gag reflex, a neuromuscular nGD issue.  But no laryngospasms, no breathing issues, nothing else.  We have dodged this bullet for now (I know it may happen in the future, but for now I’m rejoicing!).

IMPROVEMENT!!  We drove him from this appointment, and I had a HUGE smile on my face the whole way home.  It was just so unexpected.  It wasn’t that I was expecting it to be much worse (though I was expecting some worsening), but to have it significantly improve?  WOW!!!

Daddy lost his job today. After four years with the company and only three months in this dream-job position, he was let go. He is our main source of income plus our insurance was under this job. Hannah’s livelihood and survival to this point has relied exclusively on the generosity of our insurance — the multiple private therapies, orthotics, her gait trainer (which we don’t have yet), procedures, doctors, etc.

We are devastated. Our income is now based solely on my menial part-time job, which in no way could support our family, not even close. He is going to apply for unemployment tomorrow, but everyone knows that won’t even come near to what he was making. Hopefully Hannah’s medicaid will come in within the next month or so, but still —-

I don’t know what we are going to do.

Hannah's EEG at the NIH, July 2009

Hannah had her sleep-deprived EEG yesterday.

Considering she had woken up at 5:30 am on her own (she was hungry) and I had to keep her up until 1:00 pm, she did pretty well.  It was much more involved than the EEG she did at the NIH where she slept the entire time, especially when they put on the electrodes.

Unfortunately, she did NOT like the electrode placement.  She was exhausted, having someone mess with her hair with goop and gels, and she just did not want to have any of it.  But she made it through, and she went right to sleep as soon as she the technician walked out of the room.

They let her sleep for about 20 minutes or so, then they had to wake her up for another 20 minutes or so.  She did not want to be woken up, but she was somewhat willing to play toys with me in the bed.  Then they did the photic stimulation strobe light thing for about 5 minutes (talk about annoying!).

Hannah's EEG at the NIH, July 2009

The entire process was almost 2-1/2 hours.  I’m so proud of her.  She really did fantastic considering we couldn’t really explain things to her plus the fact she was just exhausted.  Surprisingly, she did not fall asleep on the way home, but she did take a nap around 6 pm for about 30 minutes, and then fell asleep for the night around 8:30 pm.

We should get the results in a week.  In my heart, I really don’t think she is having seizures.  I hope to be proven right.

We have had great luck with Hannah drinking her Compleat Pediatric formula in her bottle along.  The 4-oz formula, 4-oz whole milk or juice, and stage 2 fruit/vegetable combination has been quite successful.  Based on this meal plan at 5 bottles a day, we would use about 75 cans a month.

Unfortunately, our insurance will NOT cover this formula for us.  Why?   Because Hannah does not have a G-tube or a pump.  They would cover it if it was used in a feeding tube but NOT if it is taken by mouth.  How stupid is that?!

Cost of 75 cans a month?   About $225 a month ($75 for 24 cans)!   That is just for the formula!

Fortunately, a mom I have recently befriended gave us about 12 or so cans that her son was no longer able to use, so we have been able to give her the formula.  However, we are down to our last 3 cans (we have been using about 1 can a day).

Since our Medicaid application was never sent in, we don’t have medicaid yet even though we completed the nursing home visit.  So I am HOPING once that our Medicaid is approved, they will cover the formula cost.

Until then, we just have to suck it up and pay for it.   By the time we get to an appeal with the insurance company, we will hopefully already be on Medicaid.